We propose to evaluate the safety of the gene therapy in Limb girdle muscular dystrophy, specifically alpha-sarcoglycan deficiency, using a recombinant, replication-deficient, adeno-associated viral delivery system. The primary objective of this study is to determine the safety of adeno-associated virus (AAV) delivered via needle injection into the extensor digitorum (EDB) muscle. The EDB is a small foot muscle without important function. Muscles on both feet will be injected. One foot will receive the alpha-sarcoglycan gene and the other an injection of sterile saline. This will be done in a double-blind manner without the investigators or the patient's knowledge of which side is receiving the gene vs placebo. Six weeks after injection both EDB muscles will biopsied to determine if the gene has been effective in replacing the missing protein. This will be the first human trial of gene therapy for any type of muscular dystrophy. Currently there is no medication that is known to be effective for the treatment of any of the forms of LGMD. If this study is successful, it will represent the first step in a series of studies directed more toward efficacy of gene therapy in this disease.